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cardiomyopathy pediatric

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https://www.readbyqxmd.com/read/29238322/acute-ischemic-pediatric-stroke-management-an-extended-window-for-mechanical-thrombectomy
#1
Ashish Kulhari, Elizabeth Dorn, Jonathan Pace, Vilakshan Alambyan, Stephanie Chen, Osmond C Wu, Macym Rizvi, Anthony Hammond, Ciro Ramos-Estebanez
Ischemic stroke is a rare condition to afflict the pediatric population. Congenital cardiomyopathy represents one of several possible etiologies in children. We report a 9-year-old boy who developed right middle cerebral artery stroke secondary to primary restrictive cardiomyopathy. In the absence of pediatric guidelines, the child met adult criteria for mechanical thrombectomy given the small core infarct and large penumbra. The literature suggests children may benefit from mechanical thrombectomy in carefully selected cases...
2017: Frontiers in Neurology
https://www.readbyqxmd.com/read/29230826/evolution-of-biventricular-loading-condition-in-pediatric-lvad-patient-a-prospective-and-observational-study
#2
Arianna Di Molfetta, Roberta Iacobelli, Sergio Filippelli, Giorgia Grutter, Gianluigi Perri, Francesca Iodice, Luciano Pasquini, Paolo Guccione, Antonio Amodeo
The aim of this study was to describe the echocardiographic trend of left ventricular (LV) and right ventricular (RV) function after implantation of a pulsatile flow left ventricular assist device (LVAD) in children. From 2013 to 2016, we prospectively evaluated 13 consecutive pediatric Berlin Heart EXCOR LVAD patients. Clinical and echocardiographic data were collected at baseline, within 24 h after implantation and monthly until LVAD explant. Median age and weight at the implantation was 8 (4-23) months and 5 (4...
December 12, 2017: Artificial Organs
https://www.readbyqxmd.com/read/29212898/genetic-testing-in-pediatric-left-ventricular-noncompaction
#3
Erin M Miller, Robert B Hinton, Richard Czosek, Angela Lorts, Ashley Parrott, Amy R Shikany, Richard F Ittenbach, Stephanie M Ware
BACKGROUND: Left ventricular noncompaction (LVNC) can occur in isolation or can co-occur with a cardiomyopathy phenotype or cardiovascular malformation. The yield of cardiomyopathy gene panel testing in infants, children, and adolescents with a diagnosis of LVNC is unknown. By characterizing a pediatric population with LVNC, we sought to determine the yield of cardiomyopathy gene panel testing, distinguish the yield of testing for LVNC with or without co-occurring cardiac findings, and define additional factors influencing genetic testing yield...
December 2017: Circulation. Cardiovascular Genetics
https://www.readbyqxmd.com/read/29206935/report-of-a-myocarditis-outbreak-among-pediatric-patients-human-herpesvirus-7-as-a-causative-agent
#4
Rahmi Ozdemir, Mehmet Kucuk, Saime Ergen Dibeklioglu
Background: The etiology of myocarditis in children has not yet been completely elucidated. Objective: Medical records of eight pediatric patients diagnosed with acute myocarditis within a 41-day period in a small-town hospital were retrospectively analyzed. Methods: We examined antibody titers of adenovirus, Epstein-Barr virus, herpes simplex virus, respiratory syncytial virus, varicella-zoster virus and cytomegalovirus in peripheral blood...
November 30, 2017: Journal of Tropical Pediatrics
https://www.readbyqxmd.com/read/29188317/genetic-testing-in-pediatric-cardiomyopathy
#5
Chalani D Ellepola, Linda M Knight, Peter Fischbach, Shriprasad R Deshpande
Genetic testing is recommended in patients with dilated cardiomyopathy (DCM); however, limited studies demonstrate high yields of genetic testing in non-hypertrophic (HCM) patients. Furthermore, there is sparse genotype-phenotype data in pediatric DCM patients. We performed a retrospective review of 70 consecutive probands with cardiomyopathy (non-HCM) who underwent genetic evaluation. Mean age at presentation was 5.48 years. Echocardiography revealed mean ejection fraction of 32.4%. The LVEDd z score ranged from - 5...
November 29, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/29180164/th-17-cytokines-are-associated-with-severity-of-trypanosoma-cruzi-chronic-infection-in-pediatric-patients-from-endemic-areas-of-mexico
#6
Mariana De Alba-Alvarado, Paz María Salazar-Schettino, Luis Jiménez-Álvarez, Margarita Cabrera-Bravo, Cecilia García-Sancho, Edgar Zenteno, Clara Vazquez-Antona, Alfredo Cruz-Lagunas, Joaquín Zúñiga, Martha Irene Bucio-Torres
In Chagas disease the clinical, acute and chronic manifestations are the result of the interaction between the parasite and the host factors. The balance between inflammatory and anti-inflammatory immune responses is essential for the increase or resolution of the manifestations in individuals infected with T. cruzi. To identify if children with chronic Chagas disease and heart injury is related with non-regulated Th1, Th2 and Th17 responses. We included 31 children with T. cruzi confirmed chronic infection from endemic areas of Mexico...
November 24, 2017: Acta Tropica
https://www.readbyqxmd.com/read/29176140/role-of-whole-exome-sequencing-in-phenotype-classification-and-clinical-treatment-of-pediatric-restrictive-cardiomyopathy
#7
Wen-Hong Ding, Ling Han, Yan-Yan Xiao, Ying Mo, Jing Yang, Xiao-Fang Wang, Mei Jin
BACKGROUND: Restrictive cardiomyopathy (RCM) is the least common cardiomyopathy in which the walls are rigid and the heart is restricted from stretching and filling properly. Cardiac troponin I (cTnI) mutation-caused myofibril Ca2+ hypersensitivity has been shown to be associated with impaired diastolic function. This study aimed to investigate the linkage between the genotype and clinical therapy of RCM. METHODS: Five sporadic pediatric RCM patients confirmed by echocardiography were enrolled in this study...
December 5, 2017: Chinese Medical Journal
https://www.readbyqxmd.com/read/29174220/outcomes-of-children-supported-with-devices-labeled-as-temporary-or-short-term-a-report-from-the-pediatric-interagency-registry-for-mechanical-circulatory-support
#8
Angela Lorts, Pirooz Eghtesady, Mary Mehegan, Iki Adachi, Chet Villa, Ryan Davies, Jeffrey G Gossett, Kirk Kanter, Juan Alejos, Devin Koehl, Ryan S Cantor, David L S Morales
BACKGROUND: Historically, the "temporary" or short-term ventricular assist device (VAD) was used only as a quick bridge to recovery for children with an acute process. In the current era, the devices that were originally used for temporary support are now being used to support children for longer durations and for a variety of indications. In this study we aimed to describe the overall use, patients' characteristics and outcomes of "temporary" VAD use in children. METHODS: The Pediatric Interagency Registry for Mechanical Circulatory Support (PediMACS) is a National Institutes of Health-supported national registry for United States Food and Drug Administration-approved VADs in patients <19 years of age at the time of VAD implantation (either durable or temporary VAD)...
October 31, 2017: Journal of Heart and Lung Transplantation
https://www.readbyqxmd.com/read/29169475/outcomes-in-pediatric-dilated%C3%A2-cardiomyopathy-quo-vadis
#9
EDITORIAL
Robert G Weintraub, Peta M A Alexander
No abstract text is available yet for this article.
November 28, 2017: Journal of the American College of Cardiology
https://www.readbyqxmd.com/read/29169474/survival-without-cardiac-transplantation-among-children-with-dilated%C3%A2-cardiomyopathy
#10
Rakesh K Singh, Charles E Canter, Ling Shi, Steven D Colan, Debra A Dodd, Melanie D Everitt, Daphne T Hsu, John L Jefferies, Paul F Kantor, Elfriede Pahl, Joseph W Rossano, Jeffrey A Towbin, James D Wilkinson, Steven E Lipshultz
BACKGROUND: Studies of children with dilated cardiomyopathy (DCM) have suggested that improved survival has been primarily due to utilization of heart transplantation. OBJECTIVES: This study sought to determine transplant-free survival for these children over 20 years and identify the clinical characteristics at diagnosis that predicted death. METHODS: Children <18 years of age with some type of DCM enrolled in the Pediatric Cardiomyopathy Registry were divided by year of diagnosis into an early cohort (1990 to 1999) and a late cohort (2000 to 2009)...
November 28, 2017: Journal of the American College of Cardiology
https://www.readbyqxmd.com/read/29168549/first-polish-analysis-of-the-treatment-of-advanced-heart-failure-in-children-with-the-use-of-berlinheart-excor-mechanical-circulatory-support
#11
Szymon Pawlak, Roman Przybylski, Janusz Skalski, Joanna Śliwka, Andrzej Kansy, Adam Grzybowski, Arkadiusz Wierzyk, Jacek Białkowski, Bohdan Maruszewski, Marian Zembala
BACKGROUND: The treatment of advanced heart failure in children and infants poses a serious management problem. Heart failure in that patient group is usually of congenital etiology. The treatment schedules for pediatric patients are in most cases adapted from the guidelines for treatment of adults. Up to 2009, the treatment of that extremely difficult group of patients was limited to pharmacological therapy and occasional heart transplantations. Constantly increasing problems with recruiting donors, especially for the pediatric group, contribute to the fact that mechanical support with the use of ventricular assist devices is for many children the only chance to survive the period of waiting for a heart donor...
November 23, 2017: Kardiologia Polska
https://www.readbyqxmd.com/read/29148123/donohue-syndrome-a-review-of-literature-case-series-and-anesthetic-considerations
#12
REVIEW
Alana Kirkwood, Grant Stuart, Louise Harding
BACKGROUND: Donohue syndrome is a rare autosomal recessive disorder of insulin resistance, causing a functional defect in insulin receptor function, and affecting the ability of the insulin to bind the receptor. Features include severe hyperinsulinism and fasting hypoglycemia, along with severe failure to thrive despite feeding. An accelerated fasting state results in muscle wasting, decreased subcutaneous fat, and an excess of thick skin. A reduced thoracic diameter is accentuated by increased abdominal distension, which impacts on respiratory reserve...
November 17, 2017: Paediatric Anaesthesia
https://www.readbyqxmd.com/read/29133477/reproducibility-of-left-ventricular-dimension-versus-area-versus-volume-measurements-in-pediatric-patients-with-dilated-cardiomyopathy
#13
Elif Seda Selamet Tierney, Danielle Hollenbeck-Pringle, Caroline K Lee, Karen Altmann, Carolyn Dunbar-Masterson, Fraser Golding, Minmin Lu, Stephen G Miller, Kimberly Molina, Shobha Natarajan, Carolyn L Taylor, Felicia Trachtenberg, Steven D Colan
BACKGROUND: Multiple echocardiographic methods are used to measure left ventricular size and function. Clinical management is based on individual evaluations and longitudinal trends. The Pediatric Heart Network VVV study (Ventricular Volume Variability) in pediatric patients with dilated cardiomyopathy has reported reproducibility of several of these measures, and how disease state and number of beats impact their reproducibility. In this study, we investigated the impact of observer and sonographer variation on reproducibility of dimension, area, and volume methods to determine the best method for both individual and sequential evaluations...
November 2017: Circulation. Cardiovascular Imaging
https://www.readbyqxmd.com/read/29111293/serum-levels-of-melatonin-may-contribute-to-the-pathogenesis-of-heart-failure-in-children-with-median-age-of-1-year
#14
Yao Wu, Feifei Si, Li Luo, Qijian Yi
OBJECTIVE: Melatonin has a protective role in adults with cardiovascular disease, but the effects of melatonin in children with cardiac dysfunction are not well understood. This study was designed to explore the variations in melatonin, myeloperoxidase, and caspase-3 levels in children suffering from heart failure. METHODS: Seventy-two pediatric patients with heart failure and twelve healthy children were enrolled in this study. A modified Ross scoring system was used to evaluate clinical cardiac function...
October 28, 2017: Jornal de Pediatria
https://www.readbyqxmd.com/read/29098097/early-renal-involvement-in-a-girl-with-classic-fabry-disease
#15
Fernando Perretta, Norberto Antongiovanni, Sebastián Jaurretche
Fabry disease is an X-linked lysosomal storage disorder resulting from the deficiency or absence of the enzyme alpha galactosidase A; this defect leads to the systemic accumulation of globotriaosylceramide and its metabolites. Organic involvement in men is well known, but in women it is controversial, mainly due to the random X-chromosome inactivation in each of their cells (Lyon hypothesis). This would explain why women (heterozygotes) present a wide variability in the severity of their phenotype. The manifestations are multisystemic and begin in early childhood, reaching a severe compromise in adulthood...
2017: Case Reports in Nephrology
https://www.readbyqxmd.com/read/29079892/the-genetic-counselor-in-the-pediatric-arrhythmia-clinic-review-and-assessment-of-services
#16
Benjamin M Helm, Samantha L Freeze, Katherine G Spoonamore, Stephanie M Ware, Mark D Ayers, Adam C Kean
There are minimal data on the impact of genetic counselors in subspecialty clinics, including the pediatric arrhythmia clinic. This study aimed to describe the clinical encounters of a genetic counselor integrated into a pediatric arrhythmia clinic. In the 20 months between July 2015 and February 2017, a total of 1914 scheduled patients were screened for indications relevant for assessment by a genetic counselor. Of these, the genetic counselor completed 276 patient encounters, seeing 14.4% of all patients in clinic...
October 27, 2017: Journal of Genetic Counseling
https://www.readbyqxmd.com/read/29053178/utility-of-genetics-for-risk-stratification-in-pediatric-hypertrophic-cardiomyopathy
#17
J Mathew, L Zahavich, M Lafreniere-Roula, J Wilson, K George, L Benson, S Bowdin, S Mital
Children with hypertrophic cardiomyopathy (HCM) experience sudden cardiac death (SCD) and other life-threatening events. We assessed if affected gene and variant burden predict outcomes. Patients <18 years old with primary HCM with a pathogenic variant or variant of uncertain significance in cardiomyopathy genes were included. Association of gene and variant number and type with freedom from major adverse cardiac events (MACE) i.e. ICD insertion, myectomy, aborted SCD, transplantation or death, was assessed by Cox regression...
October 20, 2017: Clinical Genetics
https://www.readbyqxmd.com/read/29032583/third-generation-ventricular-assist-device-mid-term-outcomes-of-the-heartware-hvad-in-pediatric-patients
#18
Mustafa Pac, Sinan Sabit Kocabeyoglu, Umit Kervan, Dogan Emre Sert, Serhat Koca, Ibrahim Ece, Feyza Aysenur Pac
The HeartWare HVAD is a small, third generation continuous flow pump that is intracorporeally placed for support of a failing ventricle in adult patients. This device is small in size when compared to other left ventricular assist devices and can therefore be used in smaller sized pediatric patients. We present our initial experience using the HVAD as a bridge to heart transplantation in the pediatric population. We performed a retrospective, single center, nonrandomized review of 17 pediatric patients who underwent HVAD implantation between June 2013 and March 2016...
October 15, 2017: Artificial Organs
https://www.readbyqxmd.com/read/29019615/haemodynamic-profiles-of-children-with-end-stage-heart-failure
#19
Sharon Chen, John C Dykes, Doff B McElhinney, Robert J Gajarski, Andrew Y Shin, Seth A Hollander, Melanie E Everitt, Jack F Price, Ravi R Thiagarajan, Steven J Kindel, Joseph W Rossano, Beth D Kaufman, Lindsay J May, Elizabeth Pruitt, David N Rosenthal, Christopher S Almond
Aims: To evaluate associations between haemodynamic profiles and symptoms, end-organ function and outcome in children listed for heart transplantation. Methods and results: Children <18 years listed for heart transplant between 1993 and 2013 with cardiac catheterization data [pulmonary capillary wedge pressure (PCWP), right atrial pressure (RAP), and cardiac index (CI)] in the Pediatric Heart Transplant Study database were included. Outcomes were New York Heart Association (NYHA)/Ross classification, renal and hepatic dysfunction, and death or clinical deterioration while on waitlist...
October 7, 2017: European Heart Journal
https://www.readbyqxmd.com/read/28986271/the-subclinical-cardiomyopathy-of-friedreich-s-ataxia-in-a-pediatric-population
#20
Jonathan F Plehn, Keren Hasbani, Inez Ernst, Kenneth D Horton, Bart E Drinkard, Nicholas A Di Prospero
BACKGROUND: Identification of a subclinical cardiomyopathy in a pediatric patients with Friedreich's ataxia (FA) has not been well-described. METHODS: We performed echocardiography (echo), cardiac magnetic resonance imaging (cMRI) and neurologic assessment in a cross-sectional analysis of 48 genetically-confirmed FA subjects aged 9-17 years with moderate neurologic impairment but without a cardiovascular history. Echo and cMRI-determined left ventricular mass were indexed to height in g/m2...
October 3, 2017: Journal of Cardiac Failure
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